The aim of the study was to test the hypothesis that oral plasma cell granuloma may represent a mucosal manifestation of immunoglobulin (Ig)G4-related disease (IgG4-RD) in the oral cavity. The study sample comprised two males and four females, aged 54-79 years (median 62 years). The lesions were localized on gingival/alveolar mucosa (four cases), hard palate, and floor of the mouth, measuring 17-40 mm (median 31 mm). The duration of the lesions ranged from 3 months to several years. Information on IgG4 serum levels was available for two patients, and these were increased to 1.85 and 1.65 g/L, respectively. The follow-up period ranged 11-30 months (median 13 months). None of the lesions recurred, and none of the patients developed any manifestation of IgG4-RD. Microscopically, all cases presented as nodular lesions composed of numerous polyclonal plasma cells admixed with lymphocytes, histiocytes, mast cells, and eosinophils, set within collagenized stroma in variable proportions. Obliterative phlebitis was observed in two cases. The number of IgG4-positive plasma cells ranged between 51 and 142 per HPF (median 114), while the IgG4/IgG ratio values ranged between 0.16 and 0.72 (median 0.44) and were above 0.40 in three cases. Based on international criteria, two cases were diagnosed as definite and one as probable IgG4-RD. Oral plasma cell granuloma is a heterogeneous group of lesions, and a subset may represent a mucosal manifestation of IgG4-RD in the oral cavity.

• MeSH
• Time Factors MeSH
• Diagnosis, Differential MeSH
• Eosinophils pathology   MeSH
• Gingivitis diagnosis   immunology   pathology   MeSH
• Histiocytes pathology   MeSH
• Immunoglobulin G metabolism   MeSH
• Middle Aged MeSH
• Humans MeSH
• Mast Cells pathology   MeSH
• Immune System Diseases diagnosis   immunology   pathology   MeSH
• Mouth Diseases diagnosis   immunology   pathology   MeSH
• Plasma Cells pathology   MeSH
• Granuloma, Plasma Cell diagnosis   immunology   pathology   MeSH
• Aged MeSH
• Mouth Mucosa immunology   pathology   MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Comparative Study MeSH
• Names of Substances
• Immunoglobulin G MeSH

Retroperitoneal fibrosis (RF) is a rare disease characterized by inflammation and fibrosis of retroperitoneal soft tissues. It is classified into two types: idiopathic (iRF) and secondary (sRF). The aim of the study was to investigate the relationship between iRF and IgG4-related disease (IgG4-RD) and to eventually extend the clinicopathological features of this condition by analysis of the sample comprising six iRF and six sRF patients. The iRF patients included four males and two females, aged 12-62 years (median 55 years). Two lesions were periaortic, one was periureteral, and three cases showed both periaortic and periureteral localization. Two patients had increased serum levels of IgG4. None of the patients developed any manifestation of IgG4-RD during the follow-up period ranging for 15-133 months (median 43 months). Microscopically, in two iRF cases, fibrosis was highly cellular encircling the vessels, nerves, and paraganglia. Phlebitis was found in all cases and being obliterative in four. Lymphocytic inflammation with formation of follicles and plasma cell infiltration were scored as severe in five iRF cases. The numbers of IgG-positive plasma cells ranged 0-373 per 1 HPF (high power field; median 132) and of IgG4-positive plasma cells 0-238 per 1 HPF (median 91). The IgG4/IgG ratio values ranged 0.38-0.74 (median 0.68). Two of the iRF cases were diagnosed as definite and three cases as probable IgG4-RD. To the contrary, none of the sRF cases met the diagnostic criteria for either definite, probable, or possible IgG4-related disease. Our results indicate that a substantial portion of iRF cases, including some of very rare pediatric cases, is a manifestation of IgG4-RD.

• MeSH
• Autoimmune Diseases diagnosis   etiology   immunology   MeSH
• Child MeSH
• Adult MeSH
• Phlebitis diagnosis   immunology   pathology   MeSH
• Immunoglobulin G immunology   metabolism   MeSH
• Middle Aged MeSH
• Humans MeSH
• Plasma Cells immunology   pathology   MeSH
• Retroperitoneal Fibrosis immunology   MeSH
• Retroperitoneal Space pathology   MeSH
• Aged MeSH
• Check Tag
• Child MeSH
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Names of Substances
• Immunoglobulin G MeSH